Survival is associated with underlying ILD diagnosis in ILD-PH.
Pulmonary hypertension (PH) can complicate interstitial lung diseases (ILD) and lead to poorer outcomes. Determining prognosis factors would provide valuable information for guiding treatment decisions. We analyzed data of patients with ILD-PH from the French national prospective PH registry. Survivals were analyzed with the Kaplan-Meier method. Results are given as median [IQR]. N = 581 patients were included (male 78%, aged 70 years [64 – 77]). ILD diagnoses were idiopathic pulmonary fibrosis (IPF, n=172, 29.6%), combined pulmonary fibrosis and emphysema (CPFE, n=179, 30.8 %), hypersensitivity pneumonitis (HP, n=60, 10.3%), unclassifiable ILD (n=76, 13.1%), non-specific interstitial pneumonia (NSIP, n=53, 9.1%), pneumoconiosis (n=27, 4.6%) and other (n=14, 2.4%). Forced vital capacity was 72%pred [56 – 90], diffusing capacity for carbon monoxide was 24%pred [19-33] and pulmonary vascular resistances were 6.8 WU [5 – 9.5]. Time to lung transplantation or death was 17 months [15.3 – 19.8] in the overall population and numerically differed between patients with IPF (11.8 months [9.3 – 14.9]) and those with other ILDs (CPFE 20.9 months [17.2 – 25.3], HP 22.5 months [15.9 – 29.7], unclassifiable ILD 19.1 months [14.3 – 25.5], NSIP 15 months [12.1 – 25.9], pneumoconiosis 20.5 months [9.8 – 33.2]). <fig><object-id>erj;64/suppl_68/PA5229/F1</object-id><object-id>F1</object-id><object-id>F1</object-id><graphic></graphic></fig> In conclusion, the survival of patients with ILD-PH is related to the underlying respiratory disease.
Authors
- François Lestelle
- Bouchra Lamia (ORCID: https://orcid.org/0000-0003-2113-9813)
- Laurent Bertoletti (ORCID: https://orcid.org/0000-0001-8214-3010)
- Fabrice Bauer (ORCID: https://orcid.org/0000-0002-9221-9688)
- Nicolas Lamblin (ORCID: https://orcid.org/0000-0003-3754-1241)
- Bruno Degano (ORCID: https://orcid.org/0000-0003-1644-7264)
- Delphine Horeau-Langlard
- V. Valentin (ORCID: https://orcid.org/0000-0002-4870-3923)
- Ségolène Turquier (ORCID: https://orcid.org/0000-0001-5894-4491)
- Rémi Diesler (ORCID: https://orcid.org/0000-0003-0746-357X)
- Ari Chaouat (ORCID: https://orcid.org/0000-0001-6056-202X)
- Martine Reynaud‐Gaubert (ORCID: https://orcid.org/0000-0002-5236-5867)
- Sabrina Zeghmar
- A. Justet
- ARNAUD AB BOURDIN (ORCID: https://orcid.org/0000-0002-4645-5209)
- Cécile Tromeur (ORCID: https://orcid.org/0000-0001-9161-7521)
- Cottin Vincent
- Laurence Rottat
- Marie‐France Seronde (ORCID: https://orcid.org/0000-0003-0435-5127)
- S. Quétant
- Fabien Subtil (ORCID: https://orcid.org/0000-0003-1955-2019)
- Céline Chabanne (ORCID: https://orcid.org/0000-0001-9743-8743)
- Pierre Mauran
- Julie Traclet
- Arnaud Maurac (ORCID: https://orcid.org/0000-0003-0390-266X)
- Thomas Lacoste-Palasset (ORCID: https://orcid.org/0000-0003-3543-6675)
- Ana Nieves (ORCID: https://orcid.org/0000-0003-4825-9161)
- Nicolas Favrolt (ORCID: https://orcid.org/0000-0002-3497-3308)
- Marc Humbert (ORCID: https://orcid.org/0000-0003-0703-2892)
- Romain Trésorier
- Olivier Sitbon (ORCID: https://orcid.org/0000-0002-1942-1951)
- Frédéric Gagnadoux (ORCID: https://orcid.org/0000-0002-4231-5102)
- David Montani
- Hilario Nunes
- Sébastien Renard
- Elise Noël‐Savina
- Pascal Magro (ORCID: https://orcid.org/0000-0002-0650-9356)
- Clément Boissin
- Ibrahim Traore
Institutions
- Université Claude Bernard Lyon 1 (FR)
- Université de Bretagne Occidentale (FR)
- Université Paris-Saclay (FR)
- Centre Hospitalier Universitaire de Grenoble (FR)
- Centre Hospitalier Universitaire de Bordeaux (FR)
- Centre Hospitalier Universitaire de Lille (FR)
- Centre Hospitalier Universitaire de Nantes (FR)
- Centre Hospitalier Universitaire de Toulouse (FR)
- Sorbonne Université (FR)
- Centre Hospitalier Universitaire de Besançon (FR)
- Université Sorbonne Paris Nord (FR)
- Centre Hospitalier Universitaire de Montpellier (FR)
- Centre Hospitalier Universitaire de Tours (FR)
- Hôpital Bretonneau (FR)
- Centre Hospitalier Régional et Universitaire de Nancy (FR)
- Centre Hospitalier Universitaire de Reims (FR)
- Centre Hospitalier Universitaire d'Angers (FR)
- Centre Hospitalier Universitaire de Caen Normandie (FR)
- Hôpital Nord (FR)
- CHU Dijon Bourgogne (FR)
- Groupe Hospitalier du Havre (FR)
- Hôpital Pontchaillou (FR)
- Centre Hospitalier Universitaire de Clermont-Ferrand (FR)
- Hôpital Avicenne (FR)
- Hôpital Louis Pradel (FR)
- Centre Hospitalier Universitaire de Rouen (FR)
- Centre Hospitalier Universitaire de Saint-Étienne (FR)
- Hôpital de la Timone (FR)
- Bicêtre Hospital (FR)
- Université de Rouen Normandie (FR)
- Université Jean Monnet (FR)
- Université de Reims Champagne-Ardenne (FR)
Publication Details
- Published
- 2024-09-14
- DOI
- https://doi.org/10.1183/13993003.congress-2024.pa5229
- Primary Topic
- Systemic Sclerosis and Related Diseases
- Type
- article
- Field-Weighted Citation Impact
- 0.00